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Transplantation Trends in End Stage Hypertrophic Cardiomyopathy Over 35 Years: A UNOS Registry Analysis
Hypertrophic cardiomyopathy (HCM) is a rare but potentially life-threatening form of hereditary myopathy with increasing incidence. Therapies are emerging for HCM but the incidence of end-stage HCM has been increasing. We sought to describe the incidence and outcomes of HCM transplantation over the last 35 years.
Source: The Journal of Heart and Lung Transplantation - April 1, 2023 Category: Transplant Surgery Authors: D.J. Miklin, E. DePasquale Tags: (527) Source Type: research

Heart transplantation for COVID-19 myopathy in the United States
Evidence on characteristics and outcomes of patients undergoing heart transplantation for coronavirus disease 2019 (COVID-19) associated cardiomyopathy is limited to case reports. Of all 6332 patients aged ≥18 years undergoing heart transplant from July 2020 through May 2022 in the United Network for Organ Sharing database, 12 (0.2%) patients had COVID-19 myocarditis and 98 (1.6%) patients with the same level of care had non-COVID-19 myocarditis. Their median age was 49 (range 19-74) years. All pati ents were hospitalized in the intensive care unit and 92.7% (n=102) were on life support prior to transplantation.
Source: The Journal of Heart and Lung Transplantation - October 5, 2022 Category: Transplant Surgery Authors: George Gill, Amy Roach, Georgina Rowe, Dominic Emerson, Jon Kobashigawa, Errol P. Lobo, Fardad Esmailian, Michael E. Bowdish, Joanna Chikwe Tags: Brief Communication Source Type: research

Major Adverse Dystrophinopathy Event Score as Marker of Cumulative Morbidity and Risk for Mortality in Boys with Duchenne Muscular Dystrophy
Accurate assessments of heart failure (HF) severity in Duchenne Muscular Dystrophy (DMD) is challenging due to overlapping symptoms from cardiomyopathy, respiratory insufficiency, and skeletal myopathy. We developed an ordinal scale of multiorgan clinical variables and events graded for severity that reflect cumulative disease burden-the Major Adverse Dystrophinopathy Event (MADE) score. We hypothesized that a higher MADE score would be associated with increased mortality in DMD. We used the CINRG Duchenne Natural History Study (DNHS) dataset for MADE score validation.
Source: The Journal of Heart and Lung Transplantation - April 1, 2022 Category: Transplant Surgery Authors: B.D. Kaufman, A. Garcia, Z. He, M. Buu, C. Tesi-Rocha, J.W. Day, D.N. Rosenthal, H. Gordish-Dressman, C. Almond, T. Duong Tags: (200) Source Type: research

Successful Heart Transplantation in a Patient with Neonatal Marfan Syndrome
We describe a case of a teenage girl with NMS who developed inotrope-dependent heart failure secondary to dilated cardiomyopathy requiring heart transplantation.
Source: The Journal of Heart and Lung Transplantation - April 1, 2022 Category: Transplant Surgery Authors: S. Hussain, G. Geddes, R. Darragh, J.J. Parent Tags: (1285) Source Type: research

A Comparison of Short-Term Morbidity and Mortality Among Inpatient Lung Transplant Recipients Transplanted for COVID-19 and Other Restrictive Lung Diseases
Patients with respiratory failure (RF) who are hospitalized at the time of lung transplant (LTx) have higher post-LTx morbidity and mortality than those who are well enough to remain at home. Complications may be even worse in patients transplanted for COVID-19 (C19), as they are commonly critically ill having endured prolonged mechanical ventilation, ECMO support, myopathy, malnutrition, and superimposed infections. In a retrospective cohort study, we compared inpatient lung transplant recipients (LTxRs) transplanted for C19 vs.
Source: The Journal of Heart and Lung Transplantation - April 1, 2022 Category: Transplant Surgery Authors: D. Razia, M.T. Olson, R. Walia, R.M. Bremner, M.A. Smith, S. Tokman Tags: (289) Source Type: research

Favorable Outcomes after Heart Transplantation in Barth Syndrome
Barth Syndrome (BTHS) is a rare, X-linked disease characterized by cardioskeletal myopathy and neutropenia. Comparative outcomes after heart transplantation have not been reported.
Source: The Journal of Heart and Lung Transplantation - July 10, 2021 Category: Transplant Surgery Authors: Yu Li, Justin Godown, Carolyn L. Taylor, Anne I. Dipchand, Valerie M. Bowen, Brian Feingold Tags: Original Clinical Science Source Type: research

Immediate Post-Discharge Comprehensive Rehabilitation Program Expedites Heart Transplant Recovery and Reduces Readmission
Recovery of strength and endurance post heart transplant (HTx) has remained relatively slow. Factors that limit early rehabilitation include sternotomy precautions and corticosteroid myopathy. We propose that specialized, comprehensive rehabilitation program (COLTT) can be initiated immediately after discharge to (1) improve functional outcomes (2) decrease hospital readmission.
Source: The Journal of Heart and Lung Transplantation - March 20, 2021 Category: Transplant Surgery Authors: J. Chapa, C. DiPerna, P. Lueck, L. Tucker, C. Hage, M. Guglin, M. Jones, K. Ballut, R. Rao Tags: (627) Source Type: research

Cardiac Transplantation in Danon Disease
Danon disease is a rare X-linked dominant cardioskeletal myopathy caused by mutations in the lysosome-associated membrane protein-2 (LAMP-2) gene. Currently, there are no disease modifying therapies for Danon disease. The purpose of this study is to characterize individuals with Danon disease who undergo cardiac transplantation and describe their outcomes.
Source: The Journal of Heart and Lung Transplantation - March 20, 2021 Category: Transplant Surgery Authors: K.N. Hong, C. Battikha, A. Lin, S. John, M. Brambatti, A. Garcia-Alvarez, L. Garcia-Guereta, C. Diez, L. Perez-Gomez, P. Garcia-Pavia, M. Taylor, E. Adler Tags: (666) Source Type: research

Severe Myopathy Following Heart Transplantation in a Patient with Danon Disease
A patient with Danon disease develops a severe myopathy with rhabdomyolysis following heart transplantation (HT).
Source: The Journal of Heart and Lung Transplantation - March 20, 2021 Category: Transplant Surgery Authors: J. Guzman Bofarull, L. Castrillo, J. Milisenda, A. Garc ía Álvarez, M. Castel, E. Sandoval, T. López Sobrino, M. Farrero, F. Pérez-Villa Tags: (1236) Source Type: research

Prevalence and Outcomes of Patients with Wolff-Parkinson-White in Danon Disease: Results of a Retrospective Analysis
This study examined the prevalence of WPW and associated outcomes in a cohort of patients with Danon Disease.
Source: The Journal of Heart and Lung Transplantation - March 31, 2020 Category: Transplant Surgery Authors: M. Brooks, D. Darden, K. Hong, V. Escobedo, B. Le, C. Bougalt, E. Adler Tags: (367) Source Type: research

Heart transplantation in Danon disease: a single family displaying diverse phenotypes
Danon disease (DD) is an X-linked systemic disorder characterized by left ventricular hypertrophy, mental retardation, and skeletal myopathy.1 DD is caused by a deficiency of the lysosome-associated membrane protein (LAMP-2) which leads to an accumulation of autophagic vacuoles in skeletal and heart muscles, liver, and kidneys (Figure 1A).2 Histological analysis shows disorganized cellular architecture and hypertrophic cardiomyocytes with sarcoplasmic vacuoles. Electrocardiogram usually displays a Wolff-Parkinson-White (WPW) pre-excitation pattern.
Source: The Journal of Heart and Lung Transplantation - November 4, 2017 Category: Transplant Surgery Authors: Simon Poignant, Thierry Bourguignon, Fabien Espitalier Source Type: research

Left Ventricular Noncompaction Cardiomyopathy in Children Listed for Heart Transplant: Analysis from the Pediatric Heart Transplant Study Group
Left ventricular noncompaction cardiomyopathy (LVNC) is a rare cardiomyopathy characterized by hypertrabeculation of the left ventricle with deep intertrabecular recesses and a thin, compacted layer of myocardium. The clinical phenotype can be heterogeneous with a benign or severe course including life-threatening arrhythmias, thromboembolism, skeletal myopathy, and immune deficiencies, most notably neutropenia.1–3 Prior studies of cardiomyopathy outcomes in children listed for heart transplant (HT) did not assess patients with LVNC 4 and a recent evaluation of LVNC outcomes utilizing the United Network for Organ Sharing...
Source: The Journal of Heart and Lung Transplantation - January 6, 2016 Category: Transplant Surgery Authors: Ashwin K. Lal, Elizabeth Pruitt, Borah J. Hong, Kimberly Y. Lin, Brian Feingold Source Type: research

Left ventricular non-compaction cardiomyopathy in children listed for heart transplant: Analysis from the Pediatric Heart Transplant Study Group
Left ventricular non-compaction cardiomyopathy (LVNC) is a rare cardiomyopathy characterized by hypertrabeculation of the left ventricle with deep intertrabecular recesses and a thin, compacted layer of myocardium. The clinical phenotype can be heterogeneous with a benign or severe course, including life-threatening arrhythmias, thromboembolism, skeletal myopathy, and immune deficiencies, most notably neutropenia.1–3 Prior studies of cardiomyopathy outcomes in children listed for heart transplant (HT) did not assess patients with LVNC,4 and a recent evaluation of LVNC outcomes using the United Network for Organ Sharing d...
Source: The Journal of Heart and Lung Transplantation - January 6, 2016 Category: Transplant Surgery Authors: Ashwin K. Lal, Elizabeth Pruitt, Borah J. Hong, Kimberly Y. Lin, Brian Feingold Tags: Research Correspondence Source Type: research